Kuhara Yuta, Hashimoto Yasushi, Hirahara Satoshi, Kubota Haruna, Shirakawa Kenji, Toyota Kazuhiro, Yano Raita, Kobayashi Hironori, Yokoyama Yujiro, Sakashita Yoshihiro, Murakami Yoshiaki, Taniyama Kiyomi, Miyamoto Katsunari
Department of Surgery, Hiroshima Memorial Hospital, Honkawa-cho1-4-3, Naka-ku, Hiroshima, 730-0802, Japan.
Department of Pathology, Hiroshima Memorial Hospital, Hiroshima, Japan.
Surg Case Rep. 2021 Apr 6;7(1):81. doi: 10.1186/s40792-021-01160-4.
Metachronous pancreatic and gallbladder cancer is a rare condition and has a dismal prognosis. Herein, we present a patient with triple metachronous primary pancreatic and gallbladder cancer associated with pancreaticobiliary maljunction who achieved long-term survival after undergoing repeat curative surgery.
A 65-year-old female patient with advanced gallbladder cancer associated with pancreaticobiliary maljunction underwent extended cholecystectomy with extrahepatic bile duct resection. The pathological diagnosis was T3N0M0 stage III A papillary adenocarcinoma with hepatic invasion. During a monthly follow-up, a diffuse hypovascular 2.0 × 1.5-cm mass was detected in the pancreatic head 6.2 years after the initial surgery. Hence, the patient underwent pancreaticoduodenectomy. Histological examination revealed T3N0M0 stage IIA well-differentiated adenocarcinoma without lymph node metastases. Marked inflammatory reaction was observed in the non-cancerous lesions of the proximal pancreatic head parenchyma containing bile pigment within ductular lumens. After 12.5 years from the initial surgery, total pancreatectomy for a 4.0 × 3.0-cm mass in the remnant pancreas was performed. Histological examination revealed T3N1M0 stage IIB moderately differentiated adenocarcinoma with lymph node metastases. Hence, surgical curative resection was achieved. Based on the pathological findings, a definitive diagnosis of triple metachronous pancreatic and gallbladder cancer was made. The pathology suggests no precursor lesions such as pancreatic intraepithelial neoplasia (PanIN) and atypical flat lesions, but marked inflammations in the non-cancerous lesions, strengthening our hypothesis that chronic inflammation induced by the pancreaticobiliary maljunction is related to carcinogenesis of the pancreas. Despite further adjuvant chemotherapy, the patient's general condition worsened; however, she remained alive 15.2 years after the initial surgery while receiving the best supportive care.
Repeat curative surgery for triple metachronous cancer was associated with a favorable prognosis. Both the biliary tract and the pancreas should be closely monitored during follow-up among patients with pancreaticobiliary maljunction, which can be managed with curative surgery.
异时性胰腺和胆囊癌是一种罕见疾病，预后较差。在此，我们报告一例患有与胰胆管合流异常相关的三重异时性原发性胰腺和胆囊癌的患者，该患者在接受重复根治性手术后实现了长期生存。
一名65岁女性患者，患有与胰胆管合流异常相关的晚期胆囊癌，接受了扩大胆囊切除术及肝外胆管切除术。病理诊断为III A期T3N0M0乳头状腺癌伴肝侵犯。在每月随访期间，初次手术后6.2年，在胰头发现一个2.0×1.5厘米的弥漫性低血供肿块。因此，患者接受了胰十二指肠切除术。组织学检查显示为II A期T3N0M0高分化腺癌，无淋巴结转移。在近端胰头实质的非癌性病变中观察到明显的炎症反应，导管腔内含有胆色素。初次手术后12.5年，因残余胰腺出现一个4.0×3.0厘米的肿块而进行了全胰切除术。组织学检查显示为II B期T3N1M0中分化腺癌伴淋巴结转移。因此，实现了手术根治性切除。根据病理结果，明确诊断为三重异时性胰腺和胆囊癌。病理提示不存在胰腺上皮内瘤变（PanIN）和非典型扁平病变等前驱病变，但非癌性病变中有明显炎症，强化了我们的假设，即胰胆管合流异常引起的慢性炎症与胰腺癌发生有关。尽管进行了进一步的辅助化疗，患者的一般状况仍恶化；然而，在接受最佳支持治疗的情况下，她在初次手术后15.2年仍存活。
对三重异时性癌症进行重复根治性手术与良好的预后相关。在胰胆管合流异常患者的随访期间，应密切监测胆道和胰腺，可通过根治性手术进行处理。
